CLINICAL DETECTION OF HEPATIC DISEASE IN HEPATOLENTICULAR DEGENERATION

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Abstract
When Wilson1 described the first cases of progressive lenticular degeneration in 1912 he noted: "the most curious and the most remarkable feature of this familial disease is the constant presence of a profound degree of cirrhosis of the liver. This hepatic cirrhosis does not reveal itself by any symptoms during life; nevertheless it is always found after death." Since then all investigators2 have agreed that extensive cirrhosis of the liver is always found at autopsy in the syndrome of hepatolenticular degeneration bearing Wilson's name and that this severe hepatic involvement is a constant, essential and cardinal characteristic of the disease without which the diagnosis cannot be made. Wilson and Bruce3 and others4 expressed the belief, moreover, that hepatic cirrhosis may be the primary disease which actually precedes the lenticular lesions. Reports by Barnes and Hurst5 and Jendralski6 indicate that hepatitis may kill a patient

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